Dexmedetomidine and the perioperative care in Riley-Day syndrome: a case report and literature review

Abstract

Familial dysautonomia (FD), also known as Riley-Day syndrome, is a disorder of the autonomic nervous system
that results in loss of demyelinated nerve fibers of sensory, sympathetic and parasympathetic neurons. Individuals
with FD have variable clinical symptoms that may include insensitivity to pain, inability to produce tears, poor
oral intake during infancy, repeated vomiting, failure to thrive, wide fluctuations in body temperature, and episodic
hypertension and hypotension. These paroxysmal crises are due to dysfunction of the autonomic system with an
elevation of both norepinephrine and dopamine levels. Clonidine, an α2-adrenergic agonist, has been previously
demonstrated to be an effective pharmacological agent in the treatment of dysautonomic crises related to FD.
Dexmedetomidine is an α2-adrenergic agonist with an α2:α1 specificity that is almost 8 times that of clonidine. The
authors present the perioperative use of dexmedetomidine in a patient with FD. Previous reports of the use of
dexmedetomidine in patients with FD are reviewed and the beneficial physiologic effects discussed